Evidence-Based Management of Sickle Cell Disease Expert Panel Report, 2014 Contents Foreword ................................................................................................................................... ix Expert Panel .............................................................................................................................. xi Chapter 1: Introduction and Methodology .................................................................................. 1 Historical Perspective, Epidemiology, and Definitions ........................................................... 1 Overview of the SCD Guidelines Chapters ........................................................................... 2 Process and Methodology..................................................................................................... 3 Evidence Review and Synthesis ..................................................................................... 4 Literature Search ............................................................................................................ 5 Evidence Synthesis ......................................................................................................... 5 Evidence Framework ...................................................................................................... 6 Determining Evidence Quality ......................................................................................... 6 Determining the Strength of Recommendations .............................................................. 7 Existing Systematic Reviews and Clinical Practice Guidelines ........................................ 8 Consensus Statements ......................................................................................................... 9 Consensus–Panel Expertise ........................................................................................... 9 Consensus–Adapted ....................................................................................................... 9 Clinical Practice Guidelines and the Institute of Medicine ................................................... 10 Chapter 2: Health Maintenance for People With Sickle Cell Disease ....................................... 11 Background ........................................................................................................................ 11 Methodology ....................................................................................................................... 12 Prevention of Invasive Pneumococcal Infection .................................................................. 12 Background ................................................................................................................... 12 Key Questions ............................................................................................................... 13 Summary of the Evidence ............................................................................................. 13 Recommendations ........................................................................................................ 14 Screening for Renal Disease .............................................................................................. 14 Background ................................................................................................................... 14 Key Question ................................................................................................................ 14 Summary of the Evidence ............................................................................................. 14 Recommendations ........................................................................................................ 15 Screening for Pulmonary Hypertension ............................................................................... 15 Background ................................................................................................................... 15 Key Question ................................................................................................................ 16 Summary of the Evidence ............................................................................................. 16 Electrocardiogram Screening .............................................................................................. 17 Background ................................................................................................................... 17 Key Question ................................................................................................................ 17 Summary of the Evidence ............................................................................................. 17 Recommendations ........................................................................................................ 17 Screening for Hypertension ................................................................................................. 18 Background ................................................................................................................... 18 Key Questions ............................................................................................................... 18 Summary of the Evidence ............................................................................................. 18 Recommendations ........................................................................................................ 19 Screening for Retinopathy................................................................................................... 19 Background ................................................................................................................... 19 Key Question ................................................................................................................ 19 Summary of the Evidence ............................................................................................. 19 Recommendations ........................................................................................................ 20 EVIDENCE-BASED MANAGEMENT OF SICKLE CELL DISEASE: EXPERT PANEL REPORT, 2014 iii Screening for Risk of Stroke Using Neuroimaging .............................................................. 20 Background ................................................................................................................... 20 Key Question ................................................................................................................ 21 Summary of the Evidence ............................................................................................. 21 Recommendations ........................................................................................................ 21 Screening for Pulmonary Disease ....................................................................................... 22 Background ................................................................................................................... 22 Key Question ................................................................................................................ 22 Summary of the Evidence ............................................................................................. 22 Recommendations ........................................................................................................ 23 Reproductive Counseling .................................................................................................... 23 Background ................................................................................................................... 23 Summary of the Evidence ............................................................................................. 24 Recommendations ........................................................................................................ 24 Contraception ..................................................................................................................... 25 Background ................................................................................................................... 25 Summary of the Evidence ............................................................................................. 25 Recommendations ........................................................................................................ 26 Clinical Preventive Services ................................................................................................ 26 Background ................................................................................................................... 26 Immunizations ..................................................................................................................... 29 Background ................................................................................................................... 29 Key Question ................................................................................................................ 29 Summary of the Evidence ............................................................................................. 29 Recommendations ........................................................................................................ 29 Chapter 3: Managing Acute Complications of Sickle Cell Disease ........................................... 31 Introduction ........................................................................................................................